IED Industry Enzyme Database

Detail Information for IndEnz0018001438
IED ID IndEnz0018001438
Enzyme Type ID peroxidase001438
Protein Name Superoxide dismutase
Cu-Zn
EC 1.15.1.1
Superoxide dismutase 1
hSod1
Gene Name SOD1
Organism Homo sapiens (Human)
Taxonomic Lineage cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human)
Enzyme Sequence MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ
Enzyme Length 154
Uniprot Accession Number P00441
Absorption
Active Site
Activity Regulation
Binding Site
Calcium Binding
catalytic Activity CATALYTIC ACTIVITY: Reaction=2 H(+) + 2 superoxide = H2O2 + O2; Xref=Rhea:RHEA:20696, ChEBI:CHEBI:15378, ChEBI:CHEBI:15379, ChEBI:CHEBI:16240, ChEBI:CHEBI:18421; EC=1.15.1.1; Evidence={ECO:0000269|PubMed:24140062};
DNA Binding
EC Number 1.15.1.1
Enzyme Function FUNCTION: Destroys radicals which are normally produced within the cells and which are toxic to biological systems. {ECO:0000269|PubMed:24140062}.
Temperature Dependency
PH Dependency
Pathway
nucleotide Binding
Features Beta strand (21); Chain (1); Cross-link (1); Disulfide bond (1); Helix (4); Initiator methionine (1); Lipidation (1); Metal binding (8); Modified residue (12); Mutagenesis (15); Natural variant (81); Sequence conflict (2); Turn (2)
Keywords 3D-structure;Acetylation;Amyotrophic lateral sclerosis;Antioxidant;Copper;Cytoplasm;Direct protein sequencing;Disease variant;Disulfide bond;Lipoprotein;Metal-binding;Mitochondrion;Neurodegeneration;Nucleus;Oxidoreductase;Palmitate;Phosphoprotein;Reference proteome;Ubl conjugation;Zinc
Interact With P13928; P63010-2; Q0P5N6; Q16611; P10415; Q5SZD1; P02748; Q8N5S9-2; Q13191; O14618; P53672; P48730-2; P98082; Q8TCX1; P29692; Q8TC29; P02671-2; Q8WW76; F2Z2M7; P04406; P62879; Q9Y4H4; Q8WVV9-3; P09017; P80217-2; Q6DN90-2; O14713; Q15046; Q9NVX7-2; Q8N4N3-2; Q96JM7-2; Q9BYZ2; Q8N448; O95777; Q8TDB4; A4FUJ8; Q8N594; Q96FW1; Q6GQQ9-2; Q9BYU1; Q13113; Q08209-2; P30044; P28062-2; P62191; P17980; P43686-2; Q09028; Q6ZNA4-2; Q9ULX5; Q9NV58; Q9BUL9; P08865; Q8N488; P60896; Q15393; Q2NKQ1-4; Itself; Q3SY56; Q8NHS9; Q96MF2; Q92797-2; Q9BZK7; Q15554-4; Q9H3N1; Q13829; P07951-2; P07437; P0CG47; O75604-3; Q9GZS3; Q96NC0; O95789-4; Q8NBB4-2; Q2QGD7; P26339; P16014; P01041; P20029
Induction
Subcellular Location SUBCELLULAR LOCATION: Cytoplasm {ECO:0000269|PubMed:19741096}. Mitochondrion {ECO:0000269|PubMed:19741096}. Nucleus {ECO:0000269|PubMed:22496122}. Note=Predominantly cytoplasmic; the pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. {ECO:0000269|PubMed:19741096}.
Modified Residue MOD_RES 2; /note="N-acetylalanine"; /evidence="ECO:0000269|PubMed:1463506, ECO:0000269|PubMed:7002610, ECO:0007744|PubMed:25944712"; MOD_RES 4; /note="N6-succinyllysine"; /evidence="ECO:0000250|UniProtKB:P08228"; MOD_RES 10; /note="N6-succinyllysine"; /evidence="ECO:0000250|UniProtKB:P08228"; MOD_RES 92; /note="N6-succinyllysine"; /evidence="ECO:0000250|UniProtKB:P08228"; MOD_RES 99; /note="Phosphoserine"; /evidence="ECO:0007744|PubMed:18669648, ECO:0007744|PubMed:20068231, ECO:0007744|PubMed:24275569"; MOD_RES 103; /note="Phosphoserine"; /evidence="ECO:0007744|PubMed:24275569"; MOD_RES 106; /note="Phosphoserine"; /evidence="ECO:0000250|UniProtKB:P07632"; MOD_RES 108; /note="Phosphoserine"; /evidence="ECO:0000250|UniProtKB:P08228"; MOD_RES 123; /note="N6-acetyllysine; alternate"; /evidence="ECO:0007744|PubMed:19608861"; MOD_RES 123; /note="N6-succinyllysine; alternate"; /evidence="ECO:0000269|PubMed:24140062"; MOD_RES 137; /note="N6-acetyllysine; alternate"; /evidence="ECO:0000250|UniProtKB:P08228"; MOD_RES 137; /note="N6-succinyllysine; alternate"; /evidence="ECO:0000250|UniProtKB:P08228"
Post Translational Modification PTM: Unlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation. {ECO:0000269|PubMed:12145308, ECO:0000269|PubMed:19741096}.; PTM: The ditryptophan cross-link at Trp-33 is responsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required. {ECO:0000269|PubMed:20600836}.; PTM: Palmitoylation helps nuclear targeting and decreases catalytic activity. {ECO:0000269|PubMed:22496122}.; PTM: Succinylation, adjacent to copper catalytic site, probably inhibits activity. Desuccinylation by SIRT5 enhances activity. {ECO:0000269|PubMed:24140062}.
Signal Peptide
Structure 3D X-ray crystallography (116); NMR spectroscopy (11)
Cross Reference PDB 1AZV; 1BA9; 1DSW; 1FUN; 1HL4; 1HL5; 1KMG; 1L3N; 1MFM; 1N18; 1N19; 1OEZ; 1OZT; 1OZU; 1P1V; 1PTZ; 1PU0; 1RK7; 1SOS; 1SPD; 1UXL; 1UXM; 2AF2; 2C9S; 2C9U; 2C9V; 2GBT; 2GBU; 2GBV; 2LU5; 2MP3; 2NAM; 2NNX; 2R27; 2V0A; 2VR6; 2VR7; 2VR8; 2WKO; 2WYT; 2WYZ; 2WZ0; 2WZ5; 2WZ6; 2XJK; 2XJL; 2ZKW; 2ZKX; 2ZKY; 3CQP; 3CQQ; 3ECU; 3ECV; 3ECW; 3GQF; 3GTV; 3GZO; 3GZP; 3GZQ; 3H2P; 3H2Q; 3HFF; 3K91; 3KH3; 3KH4; 3LTV; 3QQD; 3RE0; 3T5W; 4A7G; 4A7Q; 4A7S; 4A7T; 4A7U; 4A7V; 4B3E; 4BCY; 4BCZ; 4BD4; 4FF9; 4MCM; 4MCN; 4NIN; 4NIO; 4NIP; 4OH2; 4XCR; 5DLI; 5IIW; 5J07; 5J0C; 5J0F; 5J0G; 5K02; 5O3Y; 5O40; 5U9M; 5WMJ; 5WOR; 5YTO; 5YTU; 5YUL; 6A9O; 6B79; 6DTK; 6FFK; 6FLH; 6FOI; 6FOL; 6FON; 6FP6; 6SPA; 6SPH; 6SPI; 6SPJ; 6SPK; 6Z3V; 6Z4G; 6Z4H; 6Z4I; 6Z4J; 6Z4K; 6Z4L; 6Z4M; 6Z4O; 7CJV; 7CJW;
Mapped Pubmed ID 10624810; 10631612; 11018045; 11350569; 11507097; 11561030; 11675874; 11756571; 11771762; 11854284; 11854285; 11886899; 11912919; 11930144; 11951178; 11952792; 11996514; 12039658; 12050154; 12099681; 12127151; 12138710; 12144846; 12210393; 12213295; 12215228; 12218958; 12237469; 12270693; 12356748; 12393885; 12441104; 12442272; 12447480; 12458194; 12475980; 12480087; 12485882; 12502789; 12531528; 12573532; 12590575; 12597246; 12606529; 12626552; 12644909; 12649272; 12659845; 12686560; 12710511; 12710516; 12718436; 12729761; 12732844; 12738898; 12774661; 12783432; 12787913; 12815046; 12818209; 12871577; 12871978; 12921788; 12963085; 12972170; 13129803; 13129804; 14526083; 14568129; 14578853; 14596848; 14630798; 14684739; 14690536; 14711010; 14759637; 14970233; 14978393; 14981234; 14988262; 15006704; 15016076; 15050430; 15050437; 15064408; 15068249; 15109247; 15123612; 15166009; 15193434; 15223614; 15258228; 15263088; 15288122; 15295091; 15304256; 15338334; 15350647; 15358145; 15465081; 15473258; 15474511; 15475574; 15485869; 15488469; 15507437; 15522970; 15531919; 15546588; 15576639; 15579468; 15623718; 15629867; 15634772; 15672551; 15691826; 15743779; 15753080; 15755678; 15789135; 15799963; 15812313; 15816858; 15829169; 15829914; 15851752; 15870505; 15909112; 15910777; 15961078; 15990193; 16000321; 16000635; 16020530; 16036425; 16038020; 16046140; 16046141; 16084730; 16094314; 16105836; 16132821; 16191429; 16291929; 16369483; 16407238; 16423367; 16424062; 16435343; 16467073; 16525202; 16540901; 16567804; 16583143; 16584877; 16600249; 16636275; 16644738; 16674979; 16716900; 16730180; 16741123; 16758302; 16780879; 16798882; 16857362; 16868544; 16877401; 16880213; 16895791; 16903849; 16943203; 16945901; 16952453; 16963403; 16973244; 16989689; 17005595; 17038899; 17056476; 17065353; 17077646; 17092942; 17097745; 17157186; 17157513; 17164329; 17171548; 17255946; 17257622; 17272778; 17292357; 17299743; 17328801; 17368952; 17381088; 17394531; 17418947; 17420412; 17453632; 17453634; 17457363; 17463094; 17469116; 17483589; 17504823; 17513298; 17549011; 17549529; 17555556; 17573040; 17592131; 17602442; 17624778; 17634480; 17636119; 17636481; 17646272; 17652584; 17664146; 17667843; 17683122; 17683925; 17689501; 17715066; 17725983; 17786603; 17823119; 17902702; 17903209; 17914031; 17925878; 17979159; 17987632; 17992192; 18006498; 18054242; 18055113; 18057069; 18081837; 18191946; 18192269; 18211954; 18219391; 18233996; 18239850; 18243716; 18246065; 18268245; 18273717; 18282652; 18305395; 18307039; 18316367; 18319614; 18334481; 18337461; 18384644; 18387669; 18393442; 18415766; 18423055; 18424447; 18428003; 18469020; 18476619; 18480265; 18485920; 18504130; 18515363; 18559949; 18563468; 18574763; 18579581; 18582155; 18586551; 18596033; 18608091; 18608106; 18617166; 18624915; 18636124; 18651250; 18652897; 18666828; 18676680; 18683239; 18690666; 18703498; 18712292; 18715867; 18727868; 18761352; 18775420; 18790046; 18806750; 18808763; 18826962; 18840448; 18930811; 18951903; 18971355; 19000626; 19016244; 19016906; 19022905; 19041781; 19060114; 19064571; 19077113; 19088126; 19091752; 19091790; 19109932; 19119597; 19124506; 19139308; 19156168; 19170196; 19174508; 19176896; 19187267; 19191304; 19196430; 19201909; 19227972; 19228676; 19252762; 19259395; 19266020; 19268528; 19293633; 19299510; 19325915; 19332692; 19335999; 19344917; 19363716; 19369197; 19379791; 19399611; 19399816; 19409565; 19416874; 19423521; 19423540; 19429011; 19442735; 19459941; 19478559; 19482907; 19483195; 19488901; 19497878; 19505917; 19515016; 19524271; 19527700; 19542232; 19560448; 19576169; 19596401; 19625176; 19635794; 19651777; 19654524; 19655787; 19656261; 19660548; 19661182; 19672969; 19685188; 19703565; 19705749; 19714405; 19726402; 19751676; 19779023; 19787204; 19800308; 19805546; 19805550; 19818514; 19828437; 19845829; 19878539; 19895330; 19902052; 19910193; 19913121; 19917371; 19922144; 19922450; 19949914; 19965850; 20011081; 20023586; 20052996; 20056743; 20067275; 20075587; 20084016; 20094844; 20097285; 20097856; 20105444; 20108167; 20111867; 20127819; 20151445; 20176600; 20177826; 20184519; 20184521; 20184893; 20189984; 20191117; 20195357; 20202083; 20221404; 20223753; 20226522; 20237496; 20303959; 20309572; 20346360; 20362497; 20367259; 20385105; 20399857; 20406964; 20410108; 20416077; 20438785; 20460269; 20460594; 20472325; 20477822; 20485444; 20498711; 20514411; 20515040; 20515426; 20539010; 20553075; 20553085; 20562451; 20573565; 20577002; 20595380; 20598578; 20602615; 20616033; 20628086; 20644736; 20648658; 20664914; 20682279; 20682985; 20690882; 20693657; 20709807; 20714737; 20797535; 20801718; 20808835; 20816908; 20822138; 20837142; 20838240; 20851292; 20871097; 20872977; 20877624; 20931825; 20933032; 20953194; 21056687; 21062492; 21073275; 21078175; 21083503; 21084099; 21098299; 21102999; 21120636; 21120952; 21140194; 21145892; 21151573; 21179281; 21185345; 21210247; 21226712; 21252941; 21257910; 21287393; 21325350; 21346313; 21354101; 21388680; 21393238; 21396374; 21403029; 21413851; 21421868; 21469165; 21493711; 21506602; 21515558; 21549128; 21549454; 21550164; 21554318; 21574856; 21600285; 21605634; 21620832; 21624464; 21655264; 21692800; 21700707; 21700728; 21712989; 21733494; 21760539; 21780215; 21796667; 21800301; 21816178; 21825243; 21832997; 21839474; 21843597; 21877919; 21887272; 21896300; 21908873; 21921984; 21930207; 21930926; 21940617; 21963083; 21969586; 21976959; 21986594; 22005552; 22023190; 22049684; 22072713; 22081209; 22088212; 22089180; 22094223; 22116045; 22117694; 22171159; 22185396; 22203790; 22210350; 22212531; 22214312; 22219285; 22264771; 22292843; 22292847; 22320662; 22332887; 22343163; 22357533; 22390177; 22409359; 22416121; 22424128; 22431618; 22438926; 22471402; 22471903; 22475618; 22492046; 22493728; 22542526; 22542539; 22561328; 22580443; 22589106; 22595939; 22595972; 22608880; 22609817; 22613021; 22647583; 22650006; 22651090; 22670878; 22723345; 22735487; 22761419; 22768276; 22797053; 22804629; 22853691; 22867017; 22869735; 22899264; 22910911; 22926189; 22931816; 22967507; 22970972; 22972774; 22984565; 22987392; 22999954; 23026746; 23030347; 23076148; 23076707; 23114721; 23118898; 23147108; 23147503; 23147550; 23182243; 23196439; 23264618; 23278398; 23286750; 23289810; 23291526; 23298163; 23303748; 23332757; 23339308; 23414135; 23418589; 23431152; 23431167; 23455544; 23486940; 23489186; 23496883; 23578819; 23583391; 23592792; 23597337; 23612299; 23638916; 23665075; 23720777; 23744890; 23760509; 23765103; 23781106; 23781296; 23784844; 23792044; 23795822; 23828685; 23831581; 23837654; 23840839; 23853506; 23861388; 23875973; 23889606; 23898858; 23911407; 23919400; 23927036; 23954173; 23969235; 24002644; 24023695; 24024135; 24032979; 24038157; 24040091; 24065725; 24076220; 24091828; 24094577; 24099280; 24126158; 24134191; 24143259; 24146173; 24155217; 24155874; 24184207; 24234043; 24253732; 24256636; 24269091; 24292713; 24312616; 24320678; 24325798; 24344300; 24359187; 24363822; 24369116; 24390572; 24439480; 24442855; 24448804; 24450587; 24472010; 24488615; 24493373; 24496176; 24501372; 24547927; 24550511; 24567322; 24577199; 24579824; 24591457; 24611504; 24634124; 24647101; 24692554; 24704492; 24732311; 24821155; 24838187; 24918640; 24936435; 24946209; 24947010; 24962940; 24971881; 25054289; 25093813; 25096579; 25149881; 25164820; 25167838; 25175826; 25220364; 25237191; 25283363; 25296320; 25305079; 25306968; 25316790; 25377090; 25429517; 25433341; 25434301; 25440013; 25463043; 25485927; 25496420; 25524953; 25573477; 25789959; 25792239; 25798606; 25801590; 25818327; 25825208; 25853911; 25908445; 25947891; 25998063; 26069299; 26075819; 26084641; 26099300; 26241492; 26244336; 26293199; 26298469; 26362407; 26392565; 26477504; 26496610; 26563614; 26589182; 26599430; 26600047; 26605782; 26605911; 26619801; 26619836; 26630559; 26637092; 26643113; 26674569; 26707483; 26719414; 26754593; 26756888; 26761025; 26810478; 26817806; 26823951; 26839948; 26878886; 26882452; 26926802; 26947057; 26984187; 27002158; 27002425; 27054659; 27072680; 27074676; 27121871; 27241719; 27261461; 27264826; 27378311; 27391159; 27464601; 27494151; 27496206; 27540759; 27555441; 27591900; 27603566; 27667694; 27688759; 27703255; 27721436; 27727458; 27751366; 27755600; 27783493; 27829319; 27892702; 27914828; 27974499; 27977888; 28011951; 28058676; 28096265; 28119399; 28120938; 28128448; 28132491; 28141554; 28179340; 28185644; 28222900; 28247063; 28271284; 28291249; 28294153; 28300211; 28321933; 28366453; 28403123; 28447293; 28472188; 28512644; 28533431; 28576725; 28585802; 28629863; 28680046; 28711596; 28715630; 28739857; 28750571; 28760994; 28832631; 28850080; 28859334; 28859526; 28877271; 28884318; 28899654; 28919426; 28924281; 28950184; 28965606; 28973175; 29046499; 29072670; 29157189; 29165112; 29192167; 29234142; 29369331; 29371591; 29374733; 29394243; 29409023; 29431095; 29453800; 29478603; 29483249; 29548827; 29551730; 29553298; 29649360; 29666246; 29679384; 29703933; 29748379; 29782990; 29848712; 29860398; 29861044; 29861271; 29891006; 29924645; 29935195; 30037290; 30038021; 30110532; 30142602; 30143488; 30185534; 30242181; 30255176; 30260512; 30286701; 30296255; 30300572; 30349065; 30359015; 30399166; 30401824; 30429951; 30460537; 30503815; 30622123; 30630676; 30668180; 30692571; 30701485; 30735496; 30863976; 30887850; 30909159; 30987591; 31011770; 31042755; 31157617; 31162603; 31170830; 31228093; 31230748; 31257248; 31290185; 31318169; 31323035; 31336405; 31348988; 31412164; 31415712; 31529340; 31547718; 31601079; 31625064; 31670860; 31695223; 31817166; 31830646; 31931282; 31958637; 31999698; 32005289; 32006803; 32056106; 32098365; 32112599; 32121118; 32139772; 32188708; 32188741; 32191862; 32248719; 32250500; 32422969; 32462635; 32559230; 32605607; 32617139; 32646478; 32676988; 32676989; 32701214; 32709094; 32756522; 32762033; 32767328; 32768728; 32777948; 32784718; 32818102; 32862101; 32917014; 32927603; 32948071; 33025330; 33036560; 33058881; 33218686; 33244158; 33296277; 33326235; 33329541; 33465527; 33608813; 33630959; 33636390; 33646985; 33657322; 33670299; 33737526; 33782410; 33785574; 33825682; 33859191; 34069691; 34076319; 34158851; 34247168; 34271773; 34380534; 34404558; 34653813; 34669638; 34680939; 34685754; 34711284; 34779380; 34903144; 34944061; 34946792; 34986524; 34996976; 35053410; 35123412; 35155672; 3532102; 5389100; 7021796; 8988176; 9294870; 9295278; 9726962;
Motif
Gene Encoded By
Mass 15,936
Kinetics
Metal Binding METAL 47; /note="Copper; catalytic"; /evidence="ECO:0000269|PubMed:12963370, ECO:0000269|PubMed:17548825"; METAL 49; /note="Copper; catalytic"; /evidence="ECO:0000269|PubMed:12963370, ECO:0000269|PubMed:17548825"; METAL 64; /note="Copper; catalytic"; /evidence="ECO:0000269|PubMed:12963370, ECO:0000269|PubMed:17548825"; METAL 64; /note="Zinc; via pros nitrogen"; /evidence="ECO:0000269|PubMed:20727846"; METAL 72; /note="Zinc; via pros nitrogen"; /evidence="ECO:0000269|PubMed:20727846"; METAL 81; /note="Zinc; via pros nitrogen"; /evidence="ECO:0000269|PubMed:20727846"; METAL 84; /note="Zinc; structural"; /evidence="ECO:0000269|PubMed:20727846"; METAL 121; /note="Copper; catalytic"; /evidence="ECO:0000269|PubMed:12963370, ECO:0000269|PubMed:17548825"
Rhea ID RHEA:20696
Cross Reference Brenda 1.15.1.1;